Anorectal Malformation Management of Female Patients Part II: Pediatric...
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
2 to 5% of vestibular fistulas have a vaginal septum
The ideal time to remove a vaginal septum is when the rectum is being mobilized and the perineal body is open
Women with longitudinal vaginal septa often learn to use one side of the vagina more than the other during intercourse and are often not bothered
During labor, women with longitudinal vaginal septa often blow the septum out, which can be repaired at that time
Menstrual hygiene is a major reason to remove vaginal septa - patients report needing tampons on each side or requiring both tampon and pad
Resecting a vaginal septum in an adolescent is not a difficult operation and can be done with electrocautery, getting as close to the cervix as possible without damaging it
In vestibular fistula, visual inspection of the introitus with spreading of an instrument is obligatory to look for vaginal septa
97% of vestibular fistula patients have normal vaginal anatomy without septa
For vestibular fistula with absent vagina, sigmoid colon can be used as a vaginoplasty
In absent vagina cases, the rectal fistula can be dilated without doing a colostomy, because a sigmoid colostomy would interfere with blood supply needed for sigmoid vaginoplasty
Sigmoid vaginoplasty and imperforate anus repair can be done in one stage laparoscopically with a backup colostomy
An alternative to sigmoid neovagina is to use the rectum as vagina and mobilize more proximal rectum down as neo-rectum
Using rectum as vagina should only be done if the patient is unlikely to be continent, such as with spinal anomaly or absent sacrum, because rectum has value for continence
Of 33 patients with absent vagina, 75% had urologic problems including neurogenic bladder
Of patients with absent vagina, 50% had CKD stage 3 or greater
Solitary kidneys, reflux, hydronephrosis, neurogenic bladder, and urinary tract infections are long-term sequelae in patients with absent vagina
Once absent vagina is diagnosed, aggressive screening of the urinary tract must be employed
Male ARM with any associated urologic problem requires urology collaboration
Vestibular fistula with absent vagina is an important category requiring urology collaboration because a large percentage have serious urologic problems
In absent vagina, the rectum separates nicely from the urethra with thick fibrous tissue, less adherent than rectum to posterior vagina
The ideal time to fix the vagina is when fixing the rectum because the perineal body is open
Neovagina is technically easier in younger children because the sigmoid pedicle reaches more easily when the pelvis is shorter
For premenarchal girls with vaginal septum already missed at primary repair, there is no rush to operate unless another procedure is planned
A patient can have an isolated longitudinal vaginal septum with one Müllerian system, or two cervices indicating duplicated system
Vaginoscopy to identify one versus two cervices is important in determining whether a vaginal septum represents isolated septum or duplicated Müllerian system
There are two types of cloacas: lower ones (13 cm common channel or less) and complicated ones (13 cm or greater)
Making the distinction between low and high cloacas helps avoid trouble in management
Hydrocolpos may obstruct the distal ureters and cause bilateral hydronephrosis
About 50% of cloacas have a duplicated gynecologic system
For newborn cloaca with hydrocolpos, management includes open divided colostomy and decompression of vagina with pigtail catheter rather than formal vaginostomy
Creating a colostomy can be challenging when hydrocolpos is very dilated, occasionally requiring vaginal decompression first
Attempting cystoscopy at the time of colostomy creation in cloaca makes the colostomy very difficult
For newborn cloaca, recommendation is to just divert and deal with vaginostomy without scoping at that time
Scoping the vagina can be done at 2-3 months of age with better visualization and is more pleasant than doing it in the newborn period
For large hydrocolpos that comfortably reaches the abdominal wall, a sutured tubeless vaginostomy can be done
For hydrocolpos lower than abdominal wall, ideal is tube vaginostomy using a curled tube (Pezzer or Malecot) rather than straight tube
With straight tube vaginostomy, as hydrocolpos recedes and inflammation resolves at about 2 months, the tube falls out, whereas curled tubes stay in place
Much of hydrocolpos fluid can be vaginal secretions, but much can also be urine refluxing up
Urine is often as big a problem as vaginal dilatation in hydrocolpos
Alternative to tube vaginostomy is having family intermittently catheterize the cloaca 2-3 times daily to drain urine
After the newborn period, the uterus stops secreting and most fluid in vaginal part of cloaca is urine refluxing back
As vagina distends in cloaca, it obstructs the urethra causing more urine to leak into vagina in a perpetuating cycle
Intermittent catheterization teaching should be done under ultrasound because the tube can go into right vagina, left vagina, bladder, or rectum
Without ultrasound guidance, you may go 3 days without draining the correct vagina in duplicated systems
Blind passage of catheter through perineum generally does not drain the structures you want to drain
In one case, bedside ultrasound showed very dilated hemivaginas with echogenic fluid (probably meconium and urine) and patient creatinine was about 4
Ultrasound-guided tube placement for hydrocolpos drainage can be done in the ICU
Before draining hydrocolpos, the bladder cannot be seen on ultrasound; after drainage, the bladder fills beautifully, demonstrating the physiology of ureteral compression
In most patients, draining the hydrocolpos alone is sufficient; rarely you also need to drain the bladder
In duplicated vaginal systems, both sides must be drained or only one side of hydronephrosis will improve
During colostomy opening, the dome of hydrocolpos can be opened and some septum removed to create a single chamber
If tube vaginostomy is placed and operation planned in a couple months, it may be difficult to bring the vagina down because it becomes fixed
For very large hydrocolpos, you will almost always need to be in the abdomen anyway and can take down the vaginostomy at that time
Gynecologists typically recommend teenager age for vaginal reconstruction in isolated vaginal anomalies
Laparoscopic approach for hydrocolpos management includes left upper quadrant port for visualization, right lower quadrant percutaneous vaginostomy tube, and left lower quadrant diverting colostomy